A rare case of split pons with double encephalocoele, dermal sinus tract, and lipomeningomyelocele: a case report and review of literature

Introduction

Multiple neural tube defects and pontine anomalies are relatively rare. Cases of split pons and double encephalocoeles in combination with other spinal anomalies are even rarer.

Case report

Here, we present a very rare case of split pons, twin encephalocoeles (one of which was atretic), lumbar dermal sinus tract, lumbosacral lipomeningomyelocele, thickened filum, and syringomyelia that was managed successfully at our institute.

Conclusion

Cases of split pons and double encephalocoeles in combination with other spinal anomalies are very rare, and to the best of author's knowledge, this is the first case report of these multiple anomalies in world literature.     

Intracranial Supraclinoid ICA Dissection Causing Cerebral Infarction and Subsequent Subarachnoid Hemorrhage

Background

Intracranial arterial dissection usually leads to cerebral infarction or subarachnoid hemorrhage (SAH). It is rare to see both complications in one clinical scenario.

Methods

Case report and review of the literature.

Results

A 48-year-old woman suffered a left middle cerebral infarct from a dissection of the left supraclinoid ICA. As she was recovering from the ischemic stroke 5 days later she suffered a SAH. The SAH was caused by rupture of a dissecting pseudoaneurysm, which only became evident on repeat catheter angiography. The dissecting pseudoaneurysm was treated with coil occlusion.

Conclusion

Intracranial ICA dissections are typically associated with either ischemic or hemorrhagic presentation. We report an unusual case of a patient who suffered a SAH a few days after an ischemic stroke from the dissection. This case contradicts the long-held dogma that intracranial dissection can have either an ischemic or a hemorrhagic presentation, but not both.     

Thyroid Hemorrhage Causing Airway Obstruction After Intravenous Thrombolysis for Acute Ischemic Stroke

Background

There are several life-threatening complications associated with intravenous thrombolysis after acute ischemic stroke such as symptomatic intracerebral hemorrhage, orolingual angioedema, or less frequent, bleedings of the mucosa or ecchymosis. Aside from these known critical incidents, rare and unfamiliar complications may be even more challenging, as they are unexpected and may mimic events that appear more frequently. We report a rare and unusual acute complication of intravenous thrombolysis with recombinant tissue plasminogen activator (rt-PA) (0.9 mg/kg) administered for acute ischemic stroke.

Methods

Medical records, radiologic imaging, and pathologic specimens were reviewed.

Results

A 86-year-old woman developed acute respiratory failure 20 h after thrombolysis with suspected angioedema triggered by intravenous rt-PA. The inspiratory stridor and dyspnea were unresponsive to bronchodilators, corticosteroids, and inhaled adrenaline. After endotracheal intubation, laryngoscopy showed no significant supraglottic narrowing. Thyroidal sonography and cervical computed tomography revealed a thyroidal mass causing a tracheal and vascular compression compatible with thyroidal hemorrhage. Sonography showed a nodular goiter of the right thyroid gland. A total thyroidectomy was performed and histologic analysis confirmed a hemorrhage of the right thyroidal lobe.

Conclusions

Acute airway obstruction with respiratory failure due to thyroidal hemorrhage after intravenous thrombolysis is an important life-threatening complication, mimicking an anaphylactic reaction or a more frequent orolingual angioedema.     

Gastric perforation by a ventriculoperitoneal shunt in an adult

Ventriculoperitoneal (VP) shunt operation is a common neurosurgical procedure applied for managing intracranial hydrocephalus. Migration of a distal catheter is an uncommon complication, and related gastric perforation is rarely reported. Herein, we report the case of gastric perforation in a patient with VP shunt who presented with fever. The distal catheter within the stomach was confirmed by preoperative computed tomographic scan of abdomen and gastric endoscopy. Surgical intervention and appropriate antibiotics management enabled patient recovery. This case emphasizes the importance of early recognition and surgical intervention in this rare complication related to the shunt procedure. Received 9th December 2019. Accepted 23th January 2020.

Ventriculoperitoneal (VP) shunt (VPS) operation is a common neurosurgical procedure to treat hydrocephalus related to head trauma, vascular malformation, infection, or neoplasm.1,2 Several complications associated with the VP shunt, including obstructive, kinking, infection, disconnection, pseudocyst formation, ascites, or migration, are well documented.3,4 Although the migration of peritoneal catheter can involve any intraabdominal organ, gastric perforation by VP shunt is rarely described; only approximately 20 cases have been reported thus far.2,5 Herein, we report the case of patient who underwent VPS and presented with fever thereafter. The gastric perforation by the distal catheter of the shunt was confirmed through computed tomography (CT) and gastric endoscopy. Early recognition and surgical intervention are the keystones to manage this complication. The objective of presenting this case is that early recognition and surgical intervention are the keystones to manage this complication.     

Symptomatic Cerebral Vasospasm Following Resection of a Medulloblastoma in a Child

Background

Vasospasm may occur following intracranial tumor resection but is uncommon following resection of tumors in the posterior fossa.

Methods

Case report.

Results

Here, we report an unusual pediatric case of symptomatic cerebral vasospasm following resection of a posterior fossa medulloblastoma in a 10-year-old child. CT angiogram and serial Transcranial Doppler (TCD) studies confirmed the presence of vasospasm and response to hemodynamic augmentation therapy, resulting in favorable outcome.

Conclusion

This case illustrates an unusual complication of posterior fossa tumor resection, and the potential utility of TCD studies in the detection and management of vasospasm in pediatric neurocritical care.     

Syringobulbia associated with posterior fossa meningioma: a review of the literature

Purpose

Syringomyelia can be defined as a degenerative, progressive and chronic spinal cord disease. Its association with tumors of the posterior cranial fossa (PCF) is a rare condition.

Methods

The authors report a rare case of syringobulbia consequent to a meningioma originating from PCF in a 17-year-old female, discussing the pathogenetic mechanism of development and the resolution of the syrinx cavity after surgical procedure.

Results

The postoperative period was uneventful without complications. At 6-month follow-up, MRI revealed complete tumor removal with resolution of the syrinx cavity.

Conclusions

In cases of syringomyelia and tonsillar herniation associated with PCF meningioma, the tumor resection allows to eliminate the mass effect and increases the size of the posterior fossa with the progressive ascent of the cerebellar tonsils and the consequent reduction of their downward movement with systolic pulsation. The re-establishment of a normal anatomical condition led to the gradual disappearance of syrinx and hydrocephalus.     

Paraneoplastic coma and acquired central alveolar hypoventilation as a manifestation of brainstem encephalitis in a patient with ANNA-1 antibody and small-cell lung cancer

Introduction

Paraneoplastic neurological disorders are a well recognized complication of malignancy.

Methods

A case report to expand the currently described clinical manifestations of type 1 antineuronal antibody (ANNA-1)-associated paraneoplastic encephalomyelitis to include coma.

Results

We present an unusual case of fluctuating coma and rapid fulminant progression to acute respiratory failure from central alveolar hypoventilation caused by ANNA-1 paraneoplastic encephalomyelitis associated with small-cell lung carcinoma. Paraneoplastic infiltration of the brainstem and cerebellum, including respiratory and arousal centers, was documented on autopsy.

Conclusions

Paraneoplastic encephalomyelitis should be considered as a possible cause of coma and central alveolar hypoventilation.     

Intracardiac migration of the distal catheter of ventriculoperitoneal shunt: a case report

Introduction

Migration of the distal catheter into the heart is an extremely rare complication of ventriculoperitoneal shunt, with only 11 cases reported in literature; in only four of them, there was also the migration into the pulmonary artery. The authors report the case of a young patient with this rare complication that was managed simply converting the ventriculoperitoneal shunt into a ventriculoatrial shunt.

Discussion

The migration of the distal catheter occurred through the internal jugular vein probably as the consequence of direct damage of the vessel during tunneling. Venous flow and negative intrathoracic pressure may have gradually pulled the catheter up into the heart; the upward migration may also be encouraged by the continuous movements of the patient that was agitated in the postoperative period as the consequence of her psychiatric disorder. The authors review the literature regarding migration of distal tubing into the heart and pulmonary artery. Diagnosis and management of this complication is discussed.     

Migration of a distal shunt catheter into the heart and pulmonary artery: report of a case and review of the literature

Introduction

A rare complication of ventriculoperitoneal shunt placement is the proximal migration of the distal catheter into the heart and pulmonary artery. There have been 12 reported cases regarding intracardiac migration. In five of the cases, the catheter traveled further into the pulmonary vasculature.

Case report

The authors report another case regarding the latter situation where the catheter tip localized in a right upper lobe pulmonary arterial branch. Removal of the catheter was complicated by redundancy of the catheter and a knot along the catheter. Nevertheless, extraction was successful via endovascular retrieval using interventional radiology and loop snares. Catheter migration likely began where the right internal jugular vein was penetrated during the initial shunt placement. Subsequent venous flow and negative intrathoracic pressure coordinated the proximal migration of the catheter. The authors provide a literature review to provide information regarding management of this rare complication.     

Proteus syndrome revealing itself after the treatment of a bilateral subdural haematoma

Introduction

Hypertrophy of the calvarium has different aetiologies, among them the rare Proteus syndrome.

Case report

We report here the case of a young girl initially treated for relapsing right then left large chronic subdural haematoma, who progressively developed craniofacial hypertrophy consistent with the diagnosis of Proteus syndrome. Calvarium hypertrophy was shaved and remodelled combining midface advancement, essentially for cosmetic purposes. During the first calvarium remodelling, important bleeding of the bone required large volume of blood replacement. Haemostasis workup revealed platelets aggregation anomalies. Bleeding issues during subsequent surgeries were controlled with tranexamic acid and desmopressin acetate.

Discussion

Other manifestations of Proteus syndrome, such as a right hypertrophy of the face with hypoplasia of its middle third, a pigmented epidermal nevus and asymmetric limbs and scoliosis, appeared progressively over time. Blood and fibroblast phosphatase and tensin homolog mutation was not found.

Conclusion

Literature review of operated patients with Proteus syndrome did not reveal an association with platelets anomalies. A complete haemostasis workup following this unexpected haemorrhagic complication is recommended for this rare pathology.     

CSF hydrothorax: neither migration of peritoneal catheter into the chest nor ascites. Case report and review of the literature

Introduction

Complications of ventriculoperitoneal (V/P) shunt surgery are generally due to infection, or mechanical or dynamic dysfunction. Thoracic complications like cerebrospinal fluid (CSF) hydrothorax are rarely seen.

Patient and methods

We present a CSF hydrothorax patient as a rare complication of V/P shunt surgery and review of the literature. The patient was a 7-month-old girl who had V/P shunt surgery for hydrocephalus. Six months after surgery, she was admitted to hospital with the complaint of cough. As the chest X-ray revealed hydrothorax, a chest tube was inserted. Although her shunt tip was in the abdominal cavity in shuntograms, positive beta-2 transferrin in liquid sample and Tc 99m cisternography proved that it was CSF. After we replaced her V/P shunt with a ventriculoatrial shunt, the liquid coming from the chest tube progressively diminished and disappeared, and her chest tube was removed.

Results

There are 36 CSF hydrothorax cases, including the present case, in the literature. There is peritoneal catheter migration into the chest in 22 of them (61.1?%). Half of the remaining 14 cases (38.9?%) without catheter migration have also CSF ascites. But, in the other half (seven cases), there is neither catheter migration nor CSF ascites as in the present case.

Conclusion

CSF hydrothorax following V/P shunt surgery is a very rare complication that may cause serious respiratory distress. It is important to keep in mind that peritoneal catheter migration into the chest may or may not occur. Even ascites may not accompany CSF hydrothorax in a patient without peritoneal catheter migration.     

Transorbital penetrating cerebral injury caused by a wooden stick: surgical nuances for removal of a foreign body lodged in cavernous sinus

Purpose

Penetrating head injury (PHI) is rare in civilian population and is mostly caused by low-velocity modes. A transorbital penetrating intracranial injury is very rare and more severe than traumatic brain injury.

Methods

We report a rare case of transorbital penetrating cranial injury caused by a wooden stick. The surgical strategy was planned as the wooden stick was lodged in the right cavernous sinus.

Results

The wooden stick was successfully removed. Patient made an uneventful recovery.

Conclusions

Transorbital penetrating injuries are uncommon form of injury and require a multidisciplinary approach. No attempt should be made to remove the foreign body without the backup of an operating room because of the possibility that the object may be tamponading an injured vessel. A careful planning and a strict adherence to basic perioperative principles can lead to a satisfactory outcome.     

Bilateral radial nerve compression neuropathy in the newborn

Purpose

This study aims to discuss the diagnosis and management of radial nerve compression neuropathy in the newborn.

Methods

A personal case is presented, followed by a review and analysis of clinically similar cases identified via a PubMed search of published medical literature.

Results

We report a case of a term newborn with bilateral radial neuropathy at the humerus level. Despite severe axonal involvement in the electrophysiological evaluation, the patient showed complete bilateral recovery after 3 months of follow-up.

Conclusions

Isolated radial nerve palsy is a rare event in the newborn. The condition does not require surgical treatment and usually proceeds to full and rapid spontaneous recovery.     

Pediatric microcystic meningioma: a clinical, histological, and radiographic case-based review

Background

Microcystic meningioma (MM) is a World Health Organization grade I tumor that is rare in the pediatric population. Meningiomas account for approximately 2–4 % of all childhood central nervous system (CNS) tumors compared to approximately 20 % of all adult CNS tumors. The authors present one of the few confirmed cases of microcystic meningioma in a child and discuss the characteristic radiographic appearance and histological findings.

History

We report the case of an 11-year-old boy who presented with first-time seizure and imaging consistent with brain tumor. There was significant vasogenic edema within the entire right hemisphere, disproportionate to the size of the falcine-based tumor. Histopathological analysis revealed the microcystic subtype of meningioma.

Discussion

We review the radiographic characteristics, histopathological findings, and reported pediatric cases of MM in conjunction with our case.

Conclusion

MM has distinct radiographic characteristics (variable enhancement, lack of a dural tail, and disproportionate vasogenic edema) that can be misinterpreted in the pediatric population, suggesting a more aggressive tumor.     

Perimesencephalic Subarachnoid Hemorrhage Triggered by Hypoxic Training During Swimming

Introduction

Perimesencephalic subarachnoid hemorrhage is a rare neurologic condition of unclear etiology. Multiple mechanisms have been postulated as potential triggers, but none are universally accepted.

Methods

Single observational case report and review of the literature.

Results

We describe a patient who developed perimesencephalic subarachnoid hemorrhage in the setting of “hypoxic training” (breath-holding while swimming). We describe the plausible pathophysiologic events that caused the hemorrhage.

Conclusion

The occurrence of perimesencephalic subarachnoid hemorrhage during hypoxic training suggests that acute venous congestion may be a triggering factor. The increasing popularity of hypoxic training demands vigilance from health care providers.     

Surgical management of chronic traumatic pseudomeningocele of the craniocervical junction: case report

Purpose

Chronic traumatic pseudomeningocele (PM) is a rare complication of gunshot injuries of the craniocervical junction in pediatric patients. Impairment of the CSF dynamics may cause severe symptoms and should be treated.

Methods

We report the case of a 6-year-old girl who was accidentally shot in the neck during tribal clashes. On being admitted, she was neurologically intact with cerebrospinal fluid (CSF) leakage through the wounds. She underwent primary closure of the wounds in a rural medical facility. After two episodes of meningitis, CSF leakage resolved spontaneously. Nine months later, the patient was presented with a disfiguring mass growing in the posterior neck, severe headaches, and constitutional symptoms such as loss of appetite and a failure to thrive.

Results

Neurosurgical intervention was performed with the patient in the prone position. Occipital pericranium graft was used to repair the defect, and the cavity of the PM was obliterated with muscle layers. The patient’s symptoms improved at 1 year follow-up without PM recurrence.

Conclusion

This is a rare presentation of gunshot injuries in an environment with limited neurosurgical resources. Restoring the normal pattern of CSF circulation should be the aim of any neurosurgical intervention.     

Neurological complication of non Hodgkin lymphoma in childhood: experience from a single center in Turkey

Purpose

Lymphomas are the third most common childhood malignant disease after leukemia and central nervous system (CNS) tumors. Early diagnosis of these complications will reduce mortality and morbidity. In this study we aimed to review the neurological complications of childhood non Hodgkin Lymphoma (NHL).

Patients and methods

Forty four children with NHL between 2006 and 2012 were investigated retrospectively and 14 cases with neurological complications were identified.

Results

The most common symptom was alteration of the consciousness (10 patients, 71.4 %) followed by convulsion (5 patients, 35.7 %), and hallucination (4 patients, 28.5 %); headache, eye pain, neurogenic bladder, speech disability and facial paralysis, and hemiplegia, were less common and each of them was seen in 1 (7.1 %) of the patients. The neurological complications were mostly seen in children with precursor T lymphoblastic lymphoma followed by anaplastic large cell lymphoma. The complications were secondary to medications (Eight patients) infection (two patients); CNS relapse (two patients); or CNS involvement of the primary disease (two patients). Chemotherapy-related neurologic complications were secondary to intrathecal methotrexate, l-asparaginase, vincristine, and ifosfamide

Conclusion

Advanced disease and PTLL subtype can be suggested as predictors of neurological complication. The survival rates of neurological complications are fairly good unless it is secondary to involvement of the primary disease. In patients with drug-induced neurological complications, the treatment can be safely re-administered after controlling the neurological complications. Therefore, clinicians managing children with NHL must be informative about neurological complications.     

Neurosurgical implications of Pott’s puffy tumor in children and adolescents

Introduction

Pott’s puffy tumor (PPT) is rare and usually seen as a complication of frontal sinusitis resulting in subperiosteal abscess of the frontal bone with underlying osteomyelitis. PPT is potentially severe and can lead to life-threatening intracranial complications.

Patients and methods

The authors present a series of six pediatric patients diagnosed with PPT and intracranial complications between 1999 and 2012. There were five boys and one girl, and the mean age at diagnosis was 11.8 years. All patients presented with frontal swelling, headache, and fever. Two patients had lowered level of consciousness and severe hemodynamic instability.

Results

All patients had epidural abscess, and one also presented subdural empyema. Five patients underwent drainage of all associated abscesses, bone resection, and sinusitis treatment. One was treated conservatively with broad-spectrum antibiotics, and no surgical intervention was required. All patients fully recovered their neurologic status, without further complications.     

Abdominal cerebrospinal fluid pseudocyst: a comparative analysis between children and adults

Purpose

Abdominal cerebrospinal fluid (CSF) pseudocyst is a rare but important complication in patients with ventriculoperitoneal shunt (VPS). In addition to presenting our experience, we performed a comparative analysis between children and adults with this entity. To the author’s knowledge, there are no studies in which this condition has been compared.

Methods

The PubMed database was searched for all relevant reports published from 1954 to 2012. The differences were statistically compared, especially regarding clinical investigations, etiology of the hydrocephalus, shunt revision, CSF infection, treatment, and recurrence. Chi-square test or Fisher’s exact test was used to find associations among the variables.

Results

Compiled from literature, we found 393 cases of abdominal pseudocyst: 295 children, including our cases, and 55 adults, with age not informed in 43 cases. In children, 33 % of the patients have a positive culture on presentation, with higher incidence in children younger than 10 years. In contrast, only 15 % among adults were positive CSF culture. In total, 287 abdominal pseudocyst cases who underwent shunt revision have been reported; 78.4 % of children and 62.2 % of adults. The main occurrence of this complication according to the etiology of hydrocephalus in children was different from adults. The recurrence of pseudocyst occurred in 19.8 and 24.2 % of children and adults, respectively.

Conclusions

The differences between children and adults might represent distinct trends on the etiology and treatment of this entity. Hence, additional well-designed cohort studies will be necessary to strengthen our findings.     

Diffuse calvarial cavernoma: case report and review of the literature

Introduction

Cavernous hemangiomas of the skull are very infrequent lesions. They arise from intrinsic vasculature of skull bones and manifest at the fourth to fifth decades of life. Calvarial cavernomas are very rare among lesions of the skull. These lesions are usually focal and confined to one calvarial bone.

Discussion

In this report, we are describing a child with diffuse calvarial cavernoma, a very rare lesion with distinctive clinical and radiological manifestations. This is the first case of such morphology described in the literature.